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Post–COVID-19 Acute Disseminated Encephalomyelitis in a 17-Month-Old Loren A

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Post–COVID-19 Acute Disseminated Encephalomyelitis in a 17-Month-Old Loren A Post–COVID-19 Acute Disseminated Encephalomyelitis in a 17-Month-Old Loren A. McLendon, MD,a,b Chethan K. Rao, DO, MS,a,b Cintia Carla Da Hora, MD,c Florinda Islamovic, MD,c Fernando N. Galan, MDb Neurologic manifestations of severe acute respiratory syndrome coronavirus abstract 2 (SARS-CoV-2) infection in pediatric patients have been reported in the acute and postinfectious stages of coronavirus disease 2019. Acute disseminated encephalomyelitis (ADEM) typically presents in children after a viral illness at a mean age of 3 to 7 years. A total of 60% to 90% of literature-reported pediatric patients with ADEM have minimal to no neurologic deficits at long- term follow-up. We present a 17-month-old developmentally typical girl with aDivision of Child and Adolescent Neurology, Mayo Clinic parental complaints of irritability, upper extremity weakness, and gait College of Medicine and Science, Jacksonville, Florida; disturbance. She presented to the hospital afebrile and irritable with right- bDivision of Pediatric Neurology, Nemours Children Specialty Clinic, Jacksonville, Florida; and cDepartment of Pediatrics, sided nasolabial fold flattening, neck stiffness, left upper extremity rigidity, College of Medicine - Jacksonville, University of Florida, right upper extremity paresis, bilateral lower extremity hyperreflexia, and Jacksonville, Florida truncal ataxia. During her hospital course, she became somnolent with Dr McLendon performed data acquisition, drafted autonomic instability and was transferred to intensive care. Contrasted brain the initial manuscript, revised the figure, and MRI revealed diffuse patchy T2 hyperintensities without contrast critically reviewed the manuscript; Dr Rao performed data acquisition, contributed to the enhancement. Nasopharyngeal SARS-CoV-2 polymerase chain reaction and drafting of the initial manuscript and figure, and serum antibody testing results were positive. Cerebral spinal fluid analysis critically reviewed the manuscript; Drs Da Hora and was unremarkable. Respiratory viral panel and autoimmune encephalitis and Islamovic performed data acquisition and contributed to the drafting of the initial manuscript; demyelinating disorders panel results were negative. She was started on high- Dr Galan conceptualized the study, supervised data dose methylprednisolone and intravenous immunoglobulin, with collection, and critically reviewed the manuscript; improvement in mental status, focal deficits, and ambulation. After hospital and all authors approved the final manuscript as submitted and agree to be accountable for all discharge, she received inpatient rehabilitation for 2 weeks and at 2 month aspects of the work. follow-up had a full neurologic recovery. We report the youngest case of DOI: https://doi.org/10.1542/peds.2020-049678 postinfectious ADEM due to SARS-CoV-2 in a toddler. Early recognition of Accepted for publication Mar 18, 2021 autoimmune and inflammatory complications of SARS-CoV-2 is vital for early Address correspondence to Fernando N. Galan, MD, aggressive immunomodulatory treatment and, consequently, improved Division of Child Neurology, Nemours Children morbidity in these patients. Specialty Care, 807 Children’s Way, Jacksonville, FL 32207. E-mail: [email protected] PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). Coronavirus disease 2019 (COVID-19) multisystem inflammatory syndrome, Copyright © 2021 by the American Academy of caused by the novel coronavirus severe and a variety of neurologic sequelae.2 Pediatrics acute respiratory syndrome Individual cases of seizure, FINANCIAL DISCLOSURE: The authors have indicated coronavirus 2 (SARS-CoV-2) cerebrovascular infarction, encephalitis, they have no financial relationships relevant to this disproportionately spares pediatric and myelitis in children with active or article to disclose. populations, representing ,2% of recent COVID-19 infection have been FUNDING: No external funding. 2,3 reported cases in multinational reported. POTENTIAL CONFLICT OF INTEREST: The authors have studies.1 Up to 35% of infected children indicated they have no potential conflicts of interest are asymptomatic, and the remainder Acute disseminated encephalomyelitis to disclose. often have mild symptoms, such as (ADEM), or postinfectious fever, cough, and headache. However, encephalomyelitis, is an immune-mediated To cite: McLendon LA, Rao CK, Da Hora CC, et al. severe complications related to COVID- process marked by demyelination and Post–COVID-19 Acute Disseminated – 19 in children have been reported, multifocal neurologic manifestations.4 6 Encephalomyelitis in a 17-Month-Old. Pediatrics. 2021;147(6):e2020049678 including respiratory failure, ADEM is typically preceded by symptoms Downloaded from www.aappublications.org/news by guest on September 29, 2021 PEDIATRICS Volume 147, number 6, June 2021:e2020049678 CASE REPORT of fever, malaise, vomiting, and/or difficulty feeding herself, walking, and revealed multifocal hyperintense headaches. Neurologic symptoms are sitting without support. She T2 fluid-attenuated inversion multifocal and correlate with presented to the hospital with recovery (FLAIR) signals in bilateral radiologic lesions in the brain and/or parental complaints symptoms of subcortical and periventricular white spinal cord, clinically presenting as irritability, weakness of upper matter without contrast enhancement ataxia, encephalopathy, hemiplegia, extremities, and gait disturbance. She (Fig 1). hemiparesthesias, visual changes, was found to be afebrile but irritable, seizures, or cranial nerve palsies.4,5,7,8 with subtle right-sided nasolabial fold An MRI of the whole spine without This neurologic decline occurs rapidly flattening, significant neck stiffness contrast was unremarkable. Cerebral within days to weeks after the with a positive Brudzinski’s sign, left spinal fluid (CSF) and serum studies prodromal illness; symptoms in the upper extremity rigidity, right upper were performed before the initiation majority of patients do not progress extremity paresis, bilateral lower of high-dose methylprednisolone. CSF beyond 3 months.9 We present a case extremity hyperreflexia, and truncal analysis revealed mild pleocytosis of ADEM secondary to COVID-19 in ataxia. with lymphocytic predominance: 5 a 17-month-old girl who fared well per mcL white blood cells (81% with early and aggressive In the initial evaluation, an lymphocytes, 19% monocytes), 1 per immunomodulating therapy, which electrocardiogram was included, with mcL red blood cell, glucose of 58 mg/ included high-dose corticosteroids normal sinus rhythm, possible right dL, and protein of 17 mg/dL. The CSF and intravenous immunoglobin atrium enlargement, and left had zero oligoclonal bands, with (IVIG) treatments. ventricular hypertrophy. A a normal immunoglobulin G (IgG) transthoracic echocardiogram index and IgG synthesis rate. The CSF performed in the PICU was normal. polymerase chain reaction (PCR) was CASE Laboratory evaluation noted elevated negative for enterovirus. Other A 17-month-old African American, inflammatory markers, including an infectious studies were not obtained, non-Hispanic, previously healthy, and erythrocyte sedimentation rate of given the limited CSF sample. Serum developmentally typical girl 25 mm/hour, which increased to study results for antinuclear antibody, presented to the emergency 40 mm/hour within 2 days, and Aquaporin 4 antibodies, myelin department with fatigue, lactate dehydrogenase of 406 IU/L. oligodendrocyte glycoprotein (MOG) progressively worsening weakness, Normal serum studies included antibodies, and thyroid peroxidase and unsteady gait. She had 5 days of complete blood count, comprehensive antibodies were negative. CSF and fever, ∼13 days before presentation, metabolic panels, prothrombin time serum bacterial culture results were with a maximum temperature of and international normalized ratio, negative. An upper respiratory viral 38.9°C without associated upper partial thromboplastin time, ferritin, PCR panel via nasopharyngeal swab respiratory or gastrointestinal D-dimer, brain natriuretic peptide, and a stool PCR were negative for symptoms. After fever resolution, her troponins, ammonia levels, and enterovirus and rotavirus antigen. parents noticed progressive fatigue, creatine kinase. An MRI of the brain Results from a SARS-CoV-2 PCR via decreased communication, and with and without gadolinium contrast nasopharyngeal swab and serum FIGURE 1 A, An MRI brain T2 FLAIR axial image revealing diffuse hyperintensities in the subcortical white matter. B, An MRI brain T2 axial image revealing diffuse patchy T2 hyperintensities. C, An MRI brain T2 FLAIR coronal image revealing hyperintensities in the cortical and subcortical white matter. Downloaded from www.aappublications.org/news by guest on September 29, 2021 2 MCLENDON et al SARS-CoV IgG antibody testing were postinfectious sequelae have included converting enzyme 2 receptors both positive. Infection was evaluated Guillain-Barre syndrome and ADEM. required for viral cell entry into brain by using Hologic Aptima SARS-CoV-2 Neurologic manifestations in endothelium.22 COVID-19 cases have assay (sensitivity: .90%) and serum pediatric populations mimic those been exceedingly low in pediatric SARS-CoV IgG antibody (sensitivity: seen in adult populations but also populations, but cases of serious 84%; specificity: 100%) testing include febrile seizures, acute flaccid neurologic manifestations have been performed by Abbott myelitis, and a single pediatric report reported in isolated cases.2,3,23,24
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